A strategy for glycine encephalopathy therapy.
An inherited defect in the glycine cleavage enzyme results in the condition of neonatal glycine encephalopathy which has not responded to the current innovative methods of therapy. A re-examination of the enzyme structure and metabolic pathways, leads us to recommend future clinical evaluation of (1) vitamin-responsiveness, e.g., pyridoxine, folate and lipoic acid, (2) methionine, (3) N5, N10-methylene tetrahydrofolate and (4) alpha-methylserine therapy during the critical period of neonatal brain growth and development.
D000592:Amino Acid Metabolism, Inborn Errors / Q000188:drug therapy*; D000594:Amino Acid Oxidoreductases / Q000378:metabolism; D050960:Aminomethyltransferase; D000818:Animals; D001928:Brain Diseases, Metabolic / Q000151:congenital* / Q000188:drug therapy; D002352:Carrier Proteins / Q000378:metabolism; D055598:Chemical Phenomena; D002621:Chemistry; D005998:Glycine / Q000378:metabolism*; D050841:Glycine Dehydrogenase (Decarboxylating); D006801:Humans; D019877:Hydroxymethyl and Formyl Transferases; D007223:Infant; D007231:Infant, Newborn; D008715:Methionine / Q000627:therapeutic use; D051379:Mice; D012694:Serine / Q000031:analogs & derivatives / Q000627:therapeutic use; D013763:Tetrahydrofolates / Q000627:therapeutic use; D014166:Transferases / Q000378:metabolism
D002352:Carrier Proteins; D013763:Tetrahydrofolates; C013123:5,10-methylenetetrahydrofolic acid; C035563:2-methylserine; D012694:Serine; D008715:Methionine; D000594:Amino Acid Oxidoreductases; D050841:Glycine Dehydrogenase (Decarboxylating); D014166:Transferases; D019877:Hydroxymethyl and Formyl Transferases; D050960:Aminomethyltransferase; D005998:Glycine